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Mouse Anti-ACTN2 Recombinant Antibody (V2-179643) (CBMAB-A0990-YC)

Provided herein is a Mouse monoclonal antibody against Human Actinin Alpha 2. The antibody can be used for immunoassay techniques, such as IHC, IHC-P.
See all ACTN2 antibodies
Published Data
Fig.1 Immunostaining of longitudinal sections of TA muscle from 15-wk-old WT and Apobec2−/− mice with antibodies against α-actinin.
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Fig.2 Gastrocnemius muscles were stained with α-actinin to show the Z-lines of the sarcomeres of mutant H304R/+ mice.
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Fig.3 Wertern blotting result of actinin.
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Summary

Host Animal
Mouse
Specificity
Human
Clone
V2-179643
Antibody Isotype
IgG1
Application
IHC, IHC-P

Basic Information

Immunogen
Purified rabbit skeletal alpha-actinin
Host Species
Mouse
Specificity
Human
Antibody Isotype
IgG1
Clonality
Monoclonal
Application Notes
The COA includes recommended starting dilutions, optimal dilutions should be determined by the end user.
ApplicationNote
IHC1:100
WB1:300

Formulations & Storage [For reference only, actual COA shall prevail!]

Format
Lyophilized
Buffer
1.2% sodium acetate, 2mg BSA
Preservative
0.01 mg sodium azide
Storage
Store at 4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freeze/thaw cycles.

Target

Full Name
Actinin Alpha 2
Introduction
Alpha actinins belong to the spectrin gene superfamily which represents a diverse group of cytoskeletal proteins, including the alpha and beta spectrins and dystrophins. Alpha actinin is an actin-binding protein with multiple roles in different cell types
Entrez Gene ID
88
UniProt ID
P35609
Alternative Names
Actinin Alpha 2; F-Actin Cross-Linking Protein; Alpha-Actinin Skeletal Muscle Isoform 2; Alpha-Actinin Skeletal Muscle; Alpha-Actinin-2; CMD1AA; CMH23;
Function
F-actin cross-linking protein which is thought to anchor actin to a variety of intracellular structures. This is a bundling protein.
Biological Process
Actin filament uncapping
Cardiac muscle cell development
Cell adhesion
Focal adhesion assembly
MAPK cascade
Microspike assembly
Muscle filament sliding
Negative regulation of potassium ion transmembrane transporter activity
Negative regulation of potassium ion transport
Negative regulation of protein localization to cell surface
Phospholipase C-activating angiotensin-activated signaling pathway
Platelet degranulation
Positive regulation of cation channel activity
Positive regulation of endocytic recycling
Positive regulation of potassium ion transmembrane transporter activity
Positive regulation of potassium ion transport
Protein localization to plasma membrane
Regulation of apoptotic process
Regulation of membrane potential
Regulation of NMDA receptor activity
Sarcomere organization
Cellular Location
Z line. Colocalizes with MYOZ1 and FLNC at the Z-lines of skeletal muscle.
Involvement in disease
Cardiomyopathy, familial hypertrophic 23, with or without left ventricular non-compaction (CMH23): A hereditary heart disorder characterized by ventricular hypertrophy, which is usually asymmetric and often involves the interventricular septum. The symptoms include dyspnea, syncope, collapse, palpitations, and chest pain. They can be readily provoked by exercise. The disorder has inter- and intrafamilial variability ranging from benign to malignant forms with high risk of cardiac failure and sudden cardiac death.
Cardiomyopathy, dilated 1AA, with or without left ventricular non-compaction (CMD1AA): A disorder characterized by ventricular dilation and impaired systolic function, resulting in congestive heart failure and arrhythmia. Patients are at risk of premature death.
Myopathy, congenital, with structured cores and Z-line abnormalities (MYOCOZ): An autosomal dominant muscular disorder characterized by progressive early-onset muscle weakness, gait difficulties, loss of ambulation, and respiratory insufficiency. Morphological and ultrastructural analyses of muscle biopsies reveal type 1 fiber predominance, multiple structured cores forming a circular arrangement beneath the sarcolemma, and jagged Z-lines.
Myopathy, distal, 6, adult onset, autosomal dominant (MPD6): An autosomal dominant muscular disorder characterized by adult onset of asymmetric distal muscle weakness, primarily affecting the lower limbs and resulting in gait difficulties. Some patients develop involvement of proximal and upper limb muscles.
PTM
Ubiquitinated by FBXL22, leading to proteasomal degradation.

Silviana, N. M., Andarini, S., Lyrawati, D., & Hidayat, M. (2021). Masticatory Functional Load Increases the mRNA Expression Levels of ACTN2 and ACTN3 and the Protein Expression of α-Actinin-2 in Rat Masseter Muscle. Turkish Journal of Pharmaceutical Sciences, 18(1), 28.

Lo, L. H., Lam, C. Y., To, J. C., Chiu, C. H., & Keng, V. W. (2021). Sleeping Beauty insertional mutagenesis screen identifies the pro-metastatic roles of CNPY2 and ACTN2 in hepatocellular carcinoma tumor progression. Biochemical and Biophysical Research Communications, 541, 70-77.

Sbai, O., Soussi, R., Bole, A., Khrestchatisky, M., Esclapez, M., & Ferhat, L. (2021). The actin binding protein α-actinin-2 expression is associated with dendritic spine plasticity and migrating granule cells in the rat dentate gyrus following pilocarpine-induced seizures. Experimental Neurology, 335, 113512.

Lindholm, M. E., Jimenez-Morales, D., Zhu, H., Seo, K., Amar, D., Zhao, C., ... & Wheeler, M. T. (2020). Mono-and bi-allelic protein truncating variants in alpha-actinin 2 cause cardiomyopathy through distinct mechanisms. bioRxiv.

Lindholm, M. E., Zhu, H., Seo, K., Jimenez-Morales, D., Zhao, C., Ashley, E. A., & Wheeler, M. T. (2020). Mono-and Biallelic Structural Variants in Alpha-actinin 2 Cause Contractile Dysfunction and Cardiomyopathy in Humans. Circulation, 142(Suppl_3), A14355-A14355.

Fan, L. L., Huang, H., Jin, J. Y., Li, J. J., Chen, Y. Q., & Xiang, R. (2019). Whole-exome sequencing identifies a novel mutation (p. L320R) of alpha-actinin 2 in a Chinese family with dilated cardiomyopathy and ventricular tachycardia. Cytogenetic and genome research, 157(3), 148-152.

Prondzynski, M., Lemoine, M. D., Zech, A. T., Horváth, A., Di Mauro, V., Koivumäki, J. T., ... & Carrier, L. (2019). Disease modeling of a mutation in α‐actinin 2 guides clinical therapy in hypertrophic cardiomyopathy. EMBO molecular medicine, 11(12), e11115.

Lindholm, M., Zhu, H., Huang, Y., Ashley, E. A., & Wheeler, M. (2019). NOVEL ALPHA-ACTININ 2 MUTATIONS ARE ASSOCIATED WITH CARDIOMYOPATHY AND HYPERTROPHY IN HUMAN CARDIAC TISSUE AND IPSC-DERIVED CARDIOMYOCYTES. Journal of the American College of Cardiology, 73(9S1), 1027-1027.

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For research use only. Not intended for any clinical use.

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